Scenario 3

<< Scenario 2

<Contents>

Scenario 4 >>

Scenario 3

Adult With Sickle Cell Anaemia

Mark Singer, aged 17 years, lives with his parents and his 15-year-old sister in a semi-detached house in a quiet suburban area. Mark and his sister were both born in England but his parents emigrated from Jamaica over twenty years ago. He was diagnosed as having Sickle Cell Anaemia at eighteen months following painful swelling of his hands and feet. His parents and sister have sickle cell trait and his older brother Dean, aged 18 years, died two years ago from chest syndrome, a complication of sickle cell anaemia.

Over the years, Mark has learnt to live within the limits of his illness and has coped very well in managing his symptoms. However, he has lived with the fear that, like his brother, he may die during a sickle cell related illness.

Mark has a very close school friend Jonathan, with whom he spends much of his leisure time. Jonathan is also 17 years old and is of mixed ethnic background. His mother is of Malaysian Chinese origin and his father is from a white English background. Mark is a very able student, who is currently studying for his ‘A’ level examinations. This has placed added stress upon Mark as he is hoping for good grades to enable him to study medicine at University. Mark has forgotten to drink adequately and has now become dehydrated. Whilst studying at home with Jonathan, he suddenly experienced severe abdominal and rib pain. Knowing his condition and the levels of pain associated with a sickle cell crisis, he presented along with Jonathan at the local hospital Accident & Emergency (A & E) department demanding Diamorphine. New staff on duty did not know Mark and he gave them the impression of being a drug addict. In order to get a more effective response, Mark became aggressive and more demanding, but this further convinced the medical and nursing staff that he was not in pain, but a drug user.

In desperation, Jonathan used his mobile phone to contact Delma, the Clinical Haemoglobinopathy Nurse Specialist who supports Mark and is based at the hospital. Delma immediately visited Mark, recognised the distress he was experiencing and instantly briefed the A & E staff. Their attitude changed to a more positive approach in respect to meeting Mark’s needs. A speedy assessment was undertaken by the medical registrar followed by administration of prescribed analgesia. Mark was then transferred to the medical unit for a more detailed assessment and investigations. This was followed by medical and nursing interventions involving an interdisciplinary approach i.e. Consultant Haematologist, Clinical Haemoglobinopathy Nurse Specialist, Nursing Staff and other appropriate members of the Multidisciplinary Team.

Learning activities

Main issues the scenario addresses:

	- Sickle Cell Anaemia and its effects on the body
	- Social and cultural background
	- Health practices
	- Spiritual beliefs
	- Communication issues
	- Nursing care - critically ill
	-

The issues will be explored through the following statements/questions:

	1.	How will you/the nurse meet Mark’s personal hygiene needs?
	2.	Ensure that Mark’s nutritional and hydration needs are met.
	3.	Explain the causes of sickle cell anaemia.
	4.	Facilitate the meeting of Mark’s spiritual, psychological and cultural needs whilst he is in hospital.
	5.	Encourage the family to sustain their close relationship and remain a close unit.
	6.	Assess how effectively Mark’s pain is managed.
	7.	Identify the appropriate statutory and voluntary agencies that can offer support to Mark following his discharge.

The following activities will provide the student or tutor with opportunities to engage with the above statements and questions:

(Students will undertake (a) a literature review and (b) access web pages such as those listed below to provide background information to enable them to discuss issues related to the scenario):

1.	Mark’s personal hygiene needs.
	-	Explore issues about privacy, dignity and modesty.
2.	Mark’s nutritional and hydration needs.
	-	What factors will you take into consideration about preferences and choice?
	-	What measures will you take to encourage a satisfactory intake of food and liquids?
3.	Causes of sickle cell anaemia.
	-	Describe the genetic inheritance of sickle cell anaemia with reference to the family tree.
4.	Mark’s spiritual, psychological and cultural needs whilst he is in hospital.
	-	How will you assess these needs?
	-	What factors will you consider so as not to stereotype?
5.	Sustaining close family relationship.
	-	Consider the role of family support and cohesion.
6.	Assessment of the effective management of Mark’s pain.
	-	What will you observe for and what questions might you ask?
	-	How will you confirm non-verbal signs of pain?
7.	Appropriate statutory and voluntary agencies that can offer support to Mark following his discharge.
	-	Explore the resources and their availability.
	-	Find out about the roles of statutory and voluntary agencies.

The following resources will support those learning activities:

Books

Anionwu, E.N. and Atkin, K. (2001) The Politics of Sickle Cell and Thalassaemia. Buckingham: Open University Press.

Serjeant, G.R. and Serjeant, B.E. (2001) Sickle Cell Disease. 3rd ed. Oxford: Oxford University Press.

Articles

Alleyne, J. and Thomas, V.J. (1994) The management of sickle cell crisis pain as experienced by patients and their carers. Journal of Advanced Nursing , 19, 725-732.

Atkin, K. and Ahmad, W.I.U. (2000) Living with a sickle cell disorder: how young people negotiate their care and treatment. In W.I.U.Ahmad (ed.), Ethnicity, Disability and Chronic Illness. Buckingham: Open University Press.

Atkin, K., Ahmad, W.I.U and Anionwu, E.N. (1998) Service support to families caring for a child with a sickle cell disorder or thalassaemia. Health, 2(3), 305-327.

Davies, S.C and Oni, L. (1997) Management of patients with sickle cell disease. British Medical Journal, 315, 656-660.

Maxwell, K., Streetly, A. and Bevan, D. (1999) Experiences of hospital care and treatment seeking for pain from sickle cell disease: qualitative study. British Medical Journal, 318, 1585-1590.

Nichols, R. (1996) Pain during sickle-cell crises. American Journal of Nursing, 96, 59-60.

Thomas, V. N, Wilson-Barnett, J. and Goodhart, F. (1998) The role of cognitive-behavioural therapy in the management of pain in patients with sickle cell disease. Journal of Advanced Nursing, 27(5), 1002-1009.

Tigner, R. (1998) Handling a sickle cell crisis. RN, 61,32-36. Also accessible as a self-teaching tool: www.rnweb.com/ce/798/sickle.html

Organisations

Sickle Cell Society, 54 Station Road
Harlesden, London, NW10 4UA
Tel: 020 8961 7795

Useful web resources

www.sicklecellsociety.org
A useful site for obtaining information about sickle cell disorders and trait that is aimed at lay and professional audiences. It also contains details of NHS sickle cell counselling centres and local support groups within the UK.

APoGI Project: www.chime.ucl.ac.uk/APoGI/
APoGI (Accessible publishing of genetic information) provides data on nearly all haemoglobin disorders. This includes material on sickle cell disorders and carrier states. It provides an excellent source of information.

Black Information Link (BLINK): www.blink.org.uk
A UK site for minority ethnic issues and includes informative pages on finance, business, legal matters disability, education, Europe, human rights and arts and culture. Its health information section has useful sickle cell links

The Georgia Sickle Cell Information Centre: www.emory.edu/PEDS/SICKLE
This American Website provides information for the sickle cell patient, their families as well as welfare practitioners. It is an impressive site that covers both health and social care issues.

<< Scenario 2

<Contents>

Scenario 4 >>